Pulmonary HTN

Question 1

What is the definition of pulmonary hypertension (PH)?

Answer 1

Mean pulmonary arterial pressure (mPAP) at or greater than 25 mmHg at rest.

Question 2

What are the clinical signs associated with pulmonary hypertension?

Answer 2

Progressive exertional dips, pre-syncope or syncope, exertional angina.

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Signs of right heart failure such as peripheral edema, elevated JVP, loud P2, right sided S3, right ventricular heave, holosystolic tricuspid regurg murmur.

Question 3

When patients with pulmonary hypertension start to experience presyncope or syncope, what does this say about their heart condition?

Answer 3

In pulmonary hypertension, presyncope with the Valsalva maneuver (coughing/defecation) likely reflects poor RV output rather than malignant ventricular arrhythmia.

Question 4

How is pulmonary hypertension diagnosed?

Answer 4

Right heart catheterization. A transthoracic echocardiogram can be used for non-invasive evaluation of pressures.

Question 5

Is biopsy ever indicated with pulmonary hypertension?

Answer 5

Diagnosis of pulmonary hypertension is based on clinical presentation, noninvasive tests, and hemodynamic data (eg, echocardiography). Tissue sampling typically adds no further information and carries a high risk for complications from biopsy (eg, high-pressure bleeding), so essentially, NO!

Question 6

What are the five WHO classifications of pulmonary hypertension?

Answer 6

1. Pulmonary arterial hypertension (PAH)
2. Left heart disease
3. Lung disease/chronic hypoxemia
4. Chronic thromboembolic pulmonary hypertension (CTEPH)
5. Multifactorial or unclear mechanisms.

Question 7

What is the most common cause of pulmonary hypertension?

Answer 7

WHO class III; COPD, interstitial lung disease, obstructive sleep apnea.

Question 8

If the cause for pulmonary hypertension is idiopathic, what class of pulmonary hypertension does this belong to?

Answer 8

This is class I pulmonary hypertension.

Question 9

Drugs/toxins (e.g., amphetamines), toxins, HIV, schistosomiasis, belong to which class of pulmonary hypertension?

Answer 9

Class I

Question 10

If pulmonary arterial hypertension (PAH) appears to have a familial component, which gene is implicated and what class of pulmonary hypertension does this belong to?

Answer 10

BMPR-2 mutation, Class I pulmonary hypertension.

Question 11

Connective tissue diseases (e.g., scleroderma) are associated to which class of pulmonary hypertension?

Answer 11

Class I

Question 12

What is the management of Group 1 PAH?

Answer 12

• check family history
• screen and treat underlying autoimmune disease (connective tissue disease)
• evaluate for drugs or toxins
• test for infections (schistosomiasis)
• management of HIV

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- Prescribe appropriate vasodilator:
- CCBs (diltiazem)
- Endothelin receptor antagonists (bosentan)
- PDE-5 inhibitors (sildenafil, tadalafil)
- prostacyclin agonists (selexipag)
- IV prostacyclin agonist (epoprostenol or treprostinil) for severe cases

Question 13

What causes Group 2 pulmonary hypertension?

Answer 13

Elevated left ventricular end-diastolic pressure (LVEDP) or pulmonary capillary wedge pressure (PCWP), often due to left-sided heart failure or significant valvular disease.

Question 14

How is Group 2 PH managed?

Answer 14

Perform an ECHO and optimize left ventricular function (e.g., heart failure therapy) and treat significant valve disease.

Question 15

What causes Group 3 pulmonary hypertension?

Answer 15

Chronic lung diseases (e.g., COPD, interstitial lung disease) or chronic hypoxemia (e.g., obstructive sleep apnea).

Question 16

How is Group 3 PH managed?

Answer 16

Treat the underlying respiratory condition (e.g., bronchodilators for COPD, CPAP for OSA).

Question 17

What causes Group 4 pulmonary hypertension (CTEPH)?

Answer 17

Chronic thromboembolic disease, typically after multiple pulmonary embolisms (PEs).

Question 18

How is Group 4 CTEPH managed?

Answer 18

Pulmonary thromboendarterectomy surgery (curative) and anticoagulation.

Question 19

What are the general measures for managing all forms of pulmonary hypertension?

Answer 19

Maintain normoxia, euvolemia, and sinus rhythm; contraceptive counseling; immunizations; cardiac rehabilitation.

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For all forms of pulmonary hypertension, general supportive measures aim to maintain physiologic homeostasis (ie, normoxia, euvolemia, sinus rhythm). Furthermore, the following components of care include, avoiding pregnancy due to maternal and fetal mortality remaining unacceptably high (25%-50%), even with optimal pharmacotherapy. Right ventricular (RV) failure and obstructive physiology are especially dangerous for normal peripartum volume shifts and anesthesia. Most pulmonary vasoactive medications are pregnancy class X teratogens (absolutely contraindicated). Guidelines issue a strong recommendation to avoid pregnancy, advising non-estrogen-based contraception (lower risk for venous thrombosis for women of childbearing age. Regular aerobic exercise in a supervised cardiac/pulmonary rehabilitation program, aerobic exercise, as tolerated, is safe and effective for restoring functional status. Strenuous weightlifting and other Valsalva maneuvers inhibiting venous return should be avoided. Provide nutritional support with a dietary plan to regulate salt and fluid intake and achieve weight loss or gain (eg, pulmonary cachexia), targeting a healthy BMI. Patients should be referred to an accredited PH program due to the superior outcomes. Such centers can offer psychosocial counseling and assistance to cope with the many dimensions of PH, including family planning.

Question 20

Why should women with pulmonary hypertension avoid pregnancy?

Answer 20

Pregnancy significantly increases maternal mortality (25-50%) due to peripartum volume shifts and right ventricular failure.

Question 21

What is the definitive treatment for refractory pulmonary hypertension?

Answer 21

Bilateral lung transplantation.

Question 22

What dangerous cardiac condition is secondary to chronic pulmonary hypertension?

Answer 22

Right sided heart failure, cor pulmonale.

Question 23

What is cor pulmonale?

Answer 23

Isolated right heart failure caused by advanced pulmonary hypertension due to mechanisms other than left heart failure.

Question 24

What are the clinical features of cor pulmonale?

Answer 24

Exertional dyspnea, angina, syncope, signs of right heart failure (JVD, peripheral edema), RV heave, and hepatomegaly.

Question 25

How is cor pulmonale diagnosed?

Answer 25

Right heart catheterization, which shows elevated pulmonary pressures.

Question 26

What is the gold standard for diagnosing pulmonary hypertension?

Answer 26

Right heart catheterization.

Question 27

What are common noninvasive tests for pulmonary hypertension?

Answer 27

Echocardiography, pulmonary function tests, V/Q scan, and chest imaging.

Question 28

How is cor pulmonale managed?

Answer 28

Diuretics and treatment of underlying pulmonary hypertension.

Question 29

What dangerous cardiac rhythm occurs with right sided heart failure or pulmonary hypertension?

Answer 29

Cardiac arrest in the setting of PH and RV obstruction is due to pulseless electrical activity rather than a shockable rhythm.